John H D, Thoenes W
Pathol Res Pract. 1982;173(3):236-59. doi: 10.1016/s0344-0338(82)80087-3.
The term "Endotheliotropic Hemolytic Nephroangiopathy" (EHN) comprises various clinically or pathomorphologically defined disease states with severe renal lesions (e.g. hemolytic uremic syndrome, malignant nephrosclerosis, post partum renal insufficiency) which, to date, have been considered as different entities. We attempted to assign accompanying glomerular changes based upon light and electron microscopy to the above mentioned clinical pictures and their various stages. The accordingly classified glomerular lesions (G1--G3 and Ga) are of critical importance in pathohistological differential diagnosis. Since it is assumed that fibrin is a causing event in the pathogenesis of the vascular lesions, quantitative evaluation of glomerular fibrin deposits was done. The results, when viewed with respect to time, lead to the conclusion that the microthrombotic component represents a secondary phenomenon. Thus, the primary and hence pathogenetically most important finding is a severe damage to the endothelium of the terminal renal vasculature. This endothelial damage although being expressed with variable intensity has to be regarded as the common denominator inherent to all types of glomerular lesions in EHN.
术语“嗜内皮性溶血性肾血管病”（EHN）包含各种临床或病理形态学定义的疾病状态，伴有严重的肾脏病变（如溶血尿毒综合征、恶性肾硬化、产后肾功能不全），迄今为止，这些疾病状态一直被视为不同的实体。我们试图根据光镜和电镜观察结果，将伴随的肾小球变化与上述临床症状及其不同阶段进行关联。据此分类的肾小球病变（G1 - G3和Ga）在病理组织学鉴别诊断中至关重要。由于假定纤维蛋白是血管病变发病机制中的一个致病因素，因此对肾小球纤维蛋白沉积进行了定量评估。从时间角度来看，结果表明微血栓形成成分是一种继发现象。因此，原发性且在发病机制上最重要的发现是终末肾血管内皮的严重损伤。这种内皮损伤尽管表现强度各异，但必须被视为EHN所有类型肾小球病变的共同特征。
